Pulmonary hypertension (PH) is a medical condition in which high blood pressure affects the arteries of the lungs and the right ventricle of the heart. To reduce the chance of right heart failure and death, PH must be treated promptly. While there are medications available to treat PH, in some cases surgery is required. There are several surgical options that may be recommended depending on the type of PH and the stage of heart disease.

Atrial Septostomy

For one form of PH known as pulmonary arterial hypertension (PAH), a surgical procedure called atrial septostomy may be recommended in severe cases. In this procedure, a surgeon uses a catheter to make a hole in the wall between the left and right sides of the heart. Blood can pass through the hole from the right side of the heart to the left side, thereby relieving pressure on the right side of the heart.

When Do Doctors Recommend Atrial Septostomy?

Idiopathic PAH, which occurs due to an unknown cause, may not respond well to other treatments and can progress to right heart failure. The atrial septostomy procedure may be recommended for people in such severe cases. The procedure may also be an option for those who cannot receive a lung transplant.

Outcomes of Atrial Septostomy

Because the procedure is only performed rarely in severe cases, the impact of atrial septostomy on survival is not well understood. It is not a cure, but rather is used to treat symptoms of PAH. There are risks to the procedure, so it should be performed only when the benefits outweigh the risks.

Pulmonary Endarterectomy

Pulmonary endarterectomy (PEA), also called pulmonary thromboendarterectomy, is a surgery used to treat a form of PH called chronic thromboembolic pulmonary hypertension (CTEPH). CTEPH is caused by large blood clots in the pulmonary arteries of the lungs, often the result of a pulmonary embolism.

PEA is used to remove those blood clots and restore blood flow. During the procedure, a heart-lung machine (or cardiopulmonary bypass machine) takes over the role of the heart and lungs to keep a person alive while the heart is stopped. The surgeon then opens the pulmonary arteries and removes the blood clots that block the arteries.

Which Conditions Respond Best To Pulmonary Endarterectomy?

PEA is the primary treatment suggested to treat people with CTEPH who are eligible for surgery, although the procedure cannot be performed in all cases. The location of the blockage and certain high-risk factors will determine if a person diagnosed with CTEPH can undergo surgery.

Outcomes of Pulmonary Endarterectomy

PEA is a major heart surgery and comes with significant risks, including the risk of death. While PEA is generally successful, about 4 percent of people won’t survive the surgery. For some people, PEA has the potential to cure those diagnosed with CTEPH and eliminate the disease. Those who have PEA usually have to take Coumadin (warfarin), a blood thinner, for the remainder of their lives to prevent further blockage.

Balloon Pulmonary Angioplasty

Balloon pulmonary angioplasty (BPA) is a minimally invasive procedure used to treat pulmonary hypertension, particularly CTEPH. BPA relieves blockages by using a balloon to widen the arteries and restore blood flow to the lungs.

When Do Doctors Prescribe Balloon Pulmonary Angioplasty?

BPA is most likely to be recommended to people with CTEPH who are not eligible to receive pulmonary endarterectomy surgery.

Outcomes of Balloon Pulmonary Angioplasty

BPA can improve symptoms in people with CTEPH. However, because it is a newer procedure, the effect on survival is still not well understood. Additional research is needed to track the long-term impact of undergoing BPA.

Lung Transplantation

A lung transplant is another surgical treatment option for PH. This procedure involves replacing either one or both lungs with those of an organ donor. The donor should be a genetic match to the person receiving treatment, which can make it difficult to obtain a lung transplant.

When Is Lung Transplantation Most Successful?

Medications such as intravenous prostacyclins (like epoprostenol and treprostinil), phosphodiesterase inhibitors (like sildenafil and tadalafil), and endothelin receptor antagonists (like ambrisentan, bosentan, and macitentan) have greatly improved outcomes for people with PH — without the need for surgical intervention. However, many people with PH do not respond well to medications and may still be candidates for a lung transplant. The procedure may also be used for people with lung diseases such as chronic obstructive pulmonary disease (COPD) or pulmonary fibrosis who also have severe PH.

Outcomes of Lung Transplantation

Lung transplantation has the potential to improve survival and quality of life for people being treated for PH. As with any major surgery, there are risks such as bleeding and infection associated with a lung transplant. People who have received a lung transplant must take immunosuppressive drugs for the rest of their lives to keep their body from rejecting the donor lung. They must also attend regular follow-up appointments after the procedure to monitor any symptoms of disease or organ rejection.

Talk With Others Who Understand

On myPHteam, the social network for people with pulmonary hypertension and their loved ones, more than 37,000 members come together to ask questions, give advice, and share their stories with others who understand life with pulmonary hypertension.

Have you or a loved one had surgery for pulmonary hypertension? Share your experience in the comments below, or start a conversation by posting on your Activities page.